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CD manifests as different age\u2010dependent phenotypes and affects crucial hepatic metabolic pathways including malate\u2013aspartate\u2010shuttle, glycolysis, gluconeogenesis, de novo lipogenesis and the tricarboxylic acid and urea cycles. Although the exact pathophysiology of CD remains unclear, impaired use of glucose and fatty acids as energy sources due to NADH shuttle defects and PPAR\u03b1 downregulation, respectively, indicates evident energy deficit in CD hepatocytes. The present review summarizes current trends on available and potential treatments for CD. Baseline recommendation for CD patients is dietary management, often already present as a self\u2010selected food preference, that includes protein and fat\u2010rich food, and avoidance of excess carbohydrates. At present, liver transplantation remains the sole curative option for severe CD cases. Our extensive literature review indicated medium\u2010chain triglycerides (MCT) as the most widely used CD treatment in all age groups. MCT can effectively improve symptoms across disease phenotypes by rapidly supplying energy to the liver, restoring redox balance and inducing lipogenesis. In contrast, sodium pyruvate restored glycolysis and displayed initial preclinical promise, with however limited efficacy in adult CD patients. Ursodeoxycholic acid, nitrogen scavengers and\n                    <jats:sc>L<\/jats:sc>\n                    \u2010arginine treatments effectively address specific pathophysiological aspects such as cholestasis and hyperammonemia and are commonly administered in combination with other drugs. Finally, future possibilities including restoring redox balance, amino acid supplementation, enhancing bioenergetics, improving ureagenesis and mRNA\/DNA\u2010based gene therapy are also discussed.\n                  <\/jats:p>","DOI":"10.1002\/jimd.12768","type":"journal-article","created":{"date-parts":[[2024,7,18]],"date-time":"2024-07-18T01:44:25Z","timestamp":1721267065000},"page":"1157-1174","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["The therapeutic landscape of citrin deficiency"],"prefix":"10.1002","volume":"47","author":[{"given":"Toni","family":"Vukovi\u0107","sequence":"first","affiliation":[{"name":"University Children's Hospital Zurich and Children's Research Center University of Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li Eon","family":"Kuek","sequence":"additional","affiliation":[{"name":"Citrin Foundation  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